Characteristics of children ≤36 months of age with DIPG: A report from the international DIPG registry.
Обработка статьи · этапы, ошибки и управление
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Bartlett Allison L, Lane Adam, Chaney Brooklyn, Escorza Nancy Yanez, Black Katie, Cochrane Anne, Minturn Jane, Bartels Ute, Warren Kathy, Hansford Jordan, Ziegler David, Diez Blanca, Goldman Stewart, Packer Roger, Kieran Mark, DeWire-Schottmiller Mariko, Erker Craig, Monje-Deisseroth Michelle, Wagner Lars, Koschmann Carl, Dorris Kathleen, Shih Chie-Schin, Hassall Tim, Samson Yvan, Fisher Paul, Wang Stacie S, Tsui Karen, Sevlever Gustavo, Zhu Xiaoting, Dexheimer Phillip, Asher Anthony, Fuller Christine, Drissi Rachid, Jones Blaise, Leach James, Fouladi Maryam
Аннотация
METHODS: Patients ≤36 months at diagnosis enrolled on the International DIPG Registry (IDIPGR) with central imaging confirmation were included. Presentation, clinical course, imaging, pathology and molecular findings were analyzed.
RESULTS: Among 1183 patients in IDIPGR, 40 were eligible (median age: 29 months). Median OS was 15 months. Twelve patients (30%) were LTS, 3 (7.5%) very long-term survivors ≥5 years. Among 8 untreated patients, median OS was 2 months. Patients enrolled in the registry but excluded from our study by central radiology review or tissue diagnosis had median OS of 7 months. All but 1 LTS received radiation. Among 32 treated patients, 1-, 2-, 3-, and 5-year OS rates were 68.8%, 31.2%, 15.6% and 12.5%, respectively. LTS had longer duration of presenting symptoms (P = .018). No imaging features were predictive of outcome. Tissue and genomic data were available in 18 (45%) and 10 patients, respectively. Among 9 with known H3K27M status, 6 had a mutation.
CONCLUSIONS: Children ≤36 months demonstrated significantly more LTS, with an improved median OS of 15 months; 92% of LTS received radiation. Median OS in untreated children was 2 months, compared to 17 months for treated children. LTS had longer duration of symptoms. Excluded patients demonstrated a lower OS, contradicting the hypothesis that children ≤36 months with DIPG show improved outcomes due to misdiagnosis.